Surgery Sunday morning

We've spent the past 24 hours talking to a lot of doctors. We've gotten second opinions from other surgeons and cardiologists. They have confirmed what our own doctors have been telling us: we're running out of options and something needs to be done soon. They feel strongly that unless a surgical intervention happens, Riley will probably spend the rest of his short life in an ICU. Any medical approaches to managing the PLE aren't likely to get us home - Riley is just too sick to leave the ICU and he'll eventually succumb to an infection if he stays here. And we'd only be delaying the inevitable - a Fontan takedown or a transplant are the only proven options for eliminating the PLE.

We've agreed to a surgical Fontan takedown, and the procedure has been scheduled for 8am Sunday morning (April Fool's day?!). Hopefully Riley will be on the road to recovery by Monday, his fourth birthday.

Some technical details:

• Dr. Karl will sever the Fontan conduit connection from the pulmonary artery and attach it to the heart. He won't need to muck with the IVC or the hepatic veins - they'll stay where they are (attached to the conduit).
  
• This will return Riley to a bidirectional Glenn circulation (where he was after his second surgery).
  
• When Riley came in for the Fontan, his Glenn saturations were in the high-60s to low-70s, not suitable. To boost his oxygenation, they'll also put in an aortopulmonary shunt. This should get him into the mid-80s, close to where he'd be with the Fontan fenestration. However, his ventricle will need to work much harder and his leaky valve could get leakier.
• It's unlikely that the aortopulmonary shunt would clot over like the fenestration did - there is a 5x pressure differential between the aorta and the pulmonary artery.
• It's possible that a Fontan could be re-attempted some years into the future, although it's unlikely given Riley's history of PLE. As one doctor said, "everybody's going to be pretty nervous to reopen that box."

Riley is even grouchier today than he was yesterday (if that's possible). His numbers look good and he seemed to bounce back from the cath lab quite well. It's hard on all of us to see Riley in such a dour mood, he doesn't want to be touched, won't smile and is usually telling Mom and Dad to "stop talking." The dilaudid usually brings out the real Riley for a few minutes. We miss him and hope to bring him home soon.